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Title Scabies presenting as cutaneous nodules or malar : reports of patients with scabies surrepticius masquerading as prurigo nodularis or systemic erythematosus

Permalink https://escholarship.org/uc/item/5bn8k4vx

Journal Dermatology Online Journal, 24(9)

Authors Werbel, Tyler Hinds, Brian R Cohen, Philip R

Publication Date 2018

DOI 10.5070/D3249041414

License https://creativecommons.org/licenses/by-nc-nd/4.0/ 4.0

Peer reviewed

eScholarship.org Powered by the California Digital Library University of California Volume 24 Number 9| September 2018| Dermatology Online Journal || Case Report 24(9): 8

Scabies presenting as cutaneous nodules or malar erythema: reports of patients with scabies surrepticius masquerading as prurigo nodularis or systemic lupus erythematosus

Tyler Werbel1 MS, Brian R Hinds2 MD, Philip R Cohen2 MD Affiliations: 1School of Medicine, University of California San Diego, La Jolla, California, USA, 2Department of Dermatology, University of California San Diego, La Jolla, California, USA

Corresponding Authors: Tyler Werbel, MS, 7438 High Avenue, La Jolla, CA 92037, Tel: 352-226-0683, Email: [email protected]; Philip R. Cohen, MD, 10991 Twinleaf Court, San Diego, CA 92131, Email: [email protected]

Introduction Abstract var. hominis is a that causes Scabies surrepticius is a unifying term that represents in humans with over 300 million cases non-classical presentations of scabies mite . A patient with scabies surrepticius is per year [1]. The classical presentation is described: a man with scabies masquerading as characterized by generalized pruritus and lesions prurigo nodularis. The 91-year-old man had such as burrows usually found in the finger space metastatic prostate cancer and presented with webs [2]. However, infestations with scabies can diffuse pruritic nodules. Prurigo nodularis was present with unusual morphologies lacking typical suspected; however, the biopsy revealed scabies lesions or clinical clues to the underlying parasite. In in the . He was successfully this setting, the infestation has been referred to as treated with topical 5% cream and oral scabies surrepticius (Box 1), [3, 4]. A man whose . In addition, the features of a woman with scabies mimicking systemic lupus erythematosus are scabies mimicked prurigo nodularis is described, and summarized. The 47-year-old woman had idiopathic the features of a woman with scabies mimicking and presented with systemic lupus erythematous are reported. In malar erythema and a positive antinuclear antibody addition, the subtypes of scabies surrepticius are (titer 1:320). A diagnosis of systemic lupus summarized. erythematous was entertained until skin scraping and mineral oil preparation revealed scabies mites; she was successfully treated with oral ivermectin. In conclusion, Sarcoptes scabiei infestation can present Case Synopsis with atypical clinical morphology and an absence of A 91-year-old man presented to his primary care classical lesions such as burrows conventionally physician with a one-day history of an itchy on distributed in the interdigital web spaces, volar wrists, periumbilical area, or genitalia. Scabies his chest and arms. Acute was suspected surrepticius is a term that has been designated to and he was empirically placed on cephalexin 500mg describe these unusual presentations. Prurigo twice daily for 10 days. In addition, betamethasone nodularis and systemic lupus erythematosus can be diproprionate 0.05% ointment was prescribed twice added to the litany of conditions masquerading as daily since the lesions were also pruritic. His lesions scabies and are included amongst the guises of persisted; therefore, the topical corticosteroid scabies surrepticius. ointment was switched to mometasone 0.1% cream, he was started on a methylprednisolone dose pack, Keywords: erythematosus, incognito, lupus, mite, nodularis, and he was referred to a dermatologist for nodule, prurigo, scabies, surrepticius, systemic evaluation.

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Blistering disorders Cutaneous examination showed pruritic, excoriated, Bullous erythematous nodules on the chest, abdomen, Dermatomyositis herpetiformis-like upper back, flanks, axilla, and proximal upper

Connective tissue disease extremities (Figure 1). There were no burrows Dermatomyositis-like Systemic lupus erythematosus-like between finger webs or elsewhere on the body. Infiltrative disorders Lesions were absent below the waist, including the Langerhans cell histiocytosis-like . Urticaria pigmentosa-like Miscellaneous His left chest also demonstrated a 12×8 mm dark Incognito brown and tan, irregularly bordered, pigmented Scalp lesion (Figure 2). There were no palpable neck, Papulosquamous disorders Crusted axillary, or inguinal lymph nodes. His toenails Hidden showed white dyschromia on their surface; there -like were no burrows on the dorsal feet or toe webs. Purpuric disorders Ecchymoses His workup included not only a culture for Reactive erythema but also skin biopsies of the pruritic abdominal Urticaria nodules for hematoxylin and eosin staining and Box 1. Subtypes of scabies surrepticius direct immunofluorescence studies. The pigmented lesion on the chest was also biopsied. metastatic prostate cancer involving bones and Microscopic examination of the erythematous lymph nodes after prostatectomy ten years ago. nodule on his abdomen revealed a mixed dermal After bony involvement was discovered 18 months infiltrate composed of numerous eosinophils with prior to presentation, he started androgen lymphocytes and histiocytes; within the stratum deprivation therapy and subsequently completed corneum, mite exoskeleton was observed (Figure 3). radiation therapy for a right femoral neck metastatic The direct immunofluorescence studies were lesion. Since then, he has not developed any new negative for IgG, IgM, IgA, C3, Cq1, and fibrinogen. symptoms or pain, and his prostate specific antigen The pigmented lesion demonstrated a poorly has remained suppressed. circumscribed compound melanocytic proliferation

Figure 1. Distant A) and closer B) views of the clinical features of a scabies infestation that presented as multiple pruritic, excoriated, erythematous nodules on the abdomen (some of which are circled in purple ink), chest, upper back, flanks, axilla, and proximal upper extremities of a 91-year-old man.

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Figure 2. Distant A) and closer B) views of the clinical features of a that presented incidentally as a 12×8 mm hyperpigmented lesion (which is circled in purple ink) on the left chest of a 91-year-old man. with invasion of malignant melanocytes into the total body skin checks every 3 months for the next superficial ; there was also pagetoid spread of year and every 6 months for the subsequent 4 years. melanocytes into the overlying (Figure 4). Correlation of the clinical history and pathologic Case Discussion findings of the prurigo nodularis-like lesions Scabies is a common parasitic infection caused by established the diagnosis of not only nodular the mite Sarcoptes scabei [5]. The estimated scabies, but also scabies incognito, both subtypes of worldwide prevalence is approximately 300 million scabies surrepticius. The pigmented lesion on the cases annually [2]. Individuals with scabies chest was a pT1a melanoma with a Breslow thickness infestation usually present with generalized pruritus of 0.2mm. that typically spares the head and face and is worse The bacterial culture grew methicillin-resistant at night. Cutaneous examination usually reveals Staphylococcus aureus and Klebsiella pneumoniae. He was treated with doxycycline 100mg twice daily and ciprofloxacin 500mg twice daily for ten days. The scabies infestation was initially treated with permethrin 5% cream from neck to toe; triamcinolone 0.1% cream, applied twice daily to affected areas, was used for symptomatic treatment of the associated pruritus. However, the rash persisted; therefore, he was prescribed 12mg oral ivermectin to be taken on day one and on day eight.

Both his scabies-associated symptoms and skin lesions subsequently resolved completely. Figure 3. Pathology features of a scabetic nodular lesion from the The melanoma was excised. After confirmation of abdomen of a 91-year-old man. An intact intraepidermal mite (Sarcoptes scabiei) in cross section, characterized by a thin clear margins, the wound was repaired with a side to eosinophilic exoskeleton (arrow) is present in the stratum side closure. There has been no recurrence of his corneum. There is a lymphohistiocytic infiltrate in the dermis. H&E, scabies infestation, and he is being followed with 200×.

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burrows which most often localize to the interdigital separated by one week) has also been shown to be web spaces, but may also be present in the axillae, on as effective as permethrin [6]. Other less commonly the breasts, on the buttocks, on the elbows, on the used topical agents include , flexor surfaces of the wrists, and on the genitalia. , ivermectin, , , and There are often secondary inflammatory , [2]. pustules, excoriations, and/or vesicles in affected In contrast to the classical manifestations of scabies, areas [1]. unusual presentations can occur, mimicking other The diagnosis of a scabies infestation is typically conditions. These various subtypes of scabies have established by demonstrating the mite, its more recently been referred to as scabies excrement (scybala), or its on microscopic surrepticius (Box 1), [3, 4]. examination of a specimen obtained by skin scraping A rare presentation of scabies surrepticius, that to [6]. Alternative diagnostic techniques include skin the best of our knowledge has only previously been biopsy, burrow ink test, and polymerase chain described once, is scabies imitating systemic lupus reaction [1, 7, 8]. Several noninvasive techniques erythematosus. The patient with scabies mimicking such as , in vivo reflectance confocal systemic lupus erythematosus was a 15-year-old girl microscopy, optical coherence tomography, and who presented with mouth ulcers, pruritic malar videodermatoscopy have also been described [9]. erythema, photosensitivity, and pleuritic chest pain There are a variety of treatments available for scabies (Table 1), [10]. She was subsequently found to have infestations. Topical 5% permethrin cream is very a positive antinuclear antibody (ANA) and commonly used and is often effective. Although not normochromic, normocytic anemia [10]. We have approved by the United States Food and Drug also evaluated and treated a 47-year-old woman Administration for the treatment of scabies, oral whose scabies infestation presented as malar ivermectin 200mcg/kg in two separate doses (each erythema mimicking systemic lupus erythematosus

Figure 4. Pathology features of a 12 x 8 mm hyperpigmented lesion from the left chest of a 91-year-old man. Low A and C) and higher B) magnification views show an aberrant intraepidermal distribution of pale pigmented melanocytes both as confluent single cells (asterisks) and incipient nests (arrow) along the basal layer A). There is a confluence of single melanocytes along the dermal epidermal sin stained sections B) and Melan-A immunohistochemistry (arrows, C). C) Invasive melanoma with a dermal nest (asterisk) and single melanocytes is identified to a Breslow depth of 0.2 millimeters. H&E, A) 100×; B) 200×; and C) Melan-A immunohistochemistry, 100×.

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(Table 1). However, the correct diagnosis of scabies Secondary bacterial infections of scabies lesions was made by microscopic examination of a skin have been previously recognized. Soft tissue scraping, and her symptoms resolved with topical infections such as , , and permethrin. are most common, but progression to bacteremia with distant spread of infection is possible [27]. In Interestingly, concurrent systemic conditions one study by Steer et al., 30% of school-aged children such as systemic lupus erythematosus can alter in Fiji with scabies infestation also had a bacterial the presentation of scabies (Table 2), [11-22]. superinfection. Group A Streptococcus and/or Patients with systemic lupus erythematosus tend to Staphylococcus aureus were the most common express a more diffuse and severe scabetic infection organisms causing secondary infection and were that does not spare the head and face. They also isolated in 80% and 57% of bacterial cultures appear to develop crusted scabies at a higher rate. respectively [28]. Similar to our 91-year-old male patient, scabies can Although our male patient was referred for his also mimic prurigo nodularis. Nodular scabies was prurigo nodularis-like lesions, a complete cutaneous initially described by Ayres and Anderson in 1932 examination revealed an early melanoma and [23]. Subsequently, in 1973, Konstantinov and infection of his toenails. Therefore, Stanoeva described 136 individuals with the nodular individuals with mite infestations may subtype of scabies, representing 7% of their total unsuspectingly have concurrent neoplastic or cases. They described the lesions as red or reddish- infectious conditions that can be discovered during brown, pruritic nodules which predominantly complete cutaneous examination. Once the mite localized to the axilla and groin. Despite treatment, infestation has resolved, appropriate therapy for most lesions persisted for longer than a month and other concurrent disorders or infections can be undertaken. some up to a year. Nodular scabies is considered to be a reaction to retained mite parts and antigens since mites are not typically found in Conclusion lesions older than one month [24]. Scabies surrepticius defines the clinical subtypes of In addition, our male patient had scabies incognito. scabies in which the morphology of the clinical This subtype of scabies is characterized by alteration lesions is atypical in presentation. Our 91-year-old of the classic presentation and lesion morphology related to systemic or topical corticosteroids [25]. It nodularis; in contrast, the clinical presentation of was first described by A.L. Macmillan in 1972 in a 3- scabies in our 47-year-old female patient imitated systemic lupus erythematosus. In addition, our male month-old boy who developed diffuse scabies with scabies hundreds of raised, red burrows after being treated incognito and a bacterial superinfection. The with high potency topical corticosteroids [26]. In clinician needs a heightened awareness of the possibility of scabies surrepticius in patients with cutaneous lesions attributable to another condition was described by Ive and Marks in that do not respond to therapy as expected. The 1968 [25]. correct diagnosis may be suspected when other family members present with classic lesions of pathogenic organisms: Klebsiella and methicillin- scabies infestation or if mite, scybala, or eggs are resistant Staphylococcus aureus. Hence, our male demonstrated. Patients with scabies surrepticius typically respond promptly to therapy with topical impetiginized. Oral antibiotics were used to agents such as permethrin 5% cream, systemic drugs eliminate the bacteria from the skin lesions. such as ivermectin, or both.

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a Table 1. Characteristics of scabies surrepticius patients whose mite infestations mimic systemic lupus erythematosus .

A R Med Recu C G Hx Symps Lesion Morphology Lab Studies Dx Tx r Ref Mouth MID anemia, 15 Pruritic, erythematous, scaly plaques ulcers, + ANA, 5% 1 Ca None in the malar area, nasolabial folds, SS No [15] Photo, - anti-Sm, Per W frontal area and between the fingers PCP - anti-dsDNA 47 Malar erythema, desquamative skin TCP, Iver Gen 2 AA ITP rash on chest and arms, burrows in + ANA (titer SS & 5% No CRb prur W interdigital web spaces 1:320) Per

aAbbreviations: A, age (years); AA, African-American; Abs, antibodies; ANA, antinuclear antibody; C, case; Ca, Caucasian; CR, current report; dsDNA, double strand deoxyribonucleic acid antibody; Dx, diagnosis of scabies infestation method; G, gender; Gen prur, generalized pruritus; ITP, idiopathic thrombocytopenic purpura; Iver, ivermectin oral; Med Hx, medical history; MID, mild iron deficiency; Per, permethrin topical cream; PCP, pleuritic chest pain; Photo, photosensitivity; R, race; Recur, recurrence; Ref, reference; Sm, Smith antibody; SS, skin scrape; Symps, symptoms; TCP, thrombocytopenia; Tx, treatment; W, woman; &, and; +, positive (present); -, negative (absent); %, percent. bA 47-year-old African-American woman was admitted to the hematology service for a flare of her idiopathic thrombocytopenic purpura. Within a week of admission, and while on intravenous methylprednisolone, she developed widespread pruritus in conjunction with malar erythema and desquamative skin rash on the chest and arms. Serologic positivity for antinuclear antibody (ANA) at a titer of 1:320 was det intravenous methylprednisolone dosing was increased, but the pruritus and rash failed to improve. The dermatology service was consulted. Cutaneous examination was significant for scale in the interdigital web spaces with subtle linear, tunnel-like excavations on the finger webs. Skin scraping with mineral oil preparation showed evidence of scabies: mites, eggs, and scybala. Treatment with oral ivermectin, along with topical permethrin 5% cream, resulted in full resolution of the skin changes. No other signs or symptoms of lupus erythematosus manifested.

Table 2. Characteristics of systemic lupus erythematosus patients who develop scabies infestationa. A R Les HoF C G SSS Bur Secondary lesion morphology loc Inv Dx Tx Recur Ref 11 20% 1 Ca Crust NS Gray hyperkeratotic plaques and crusted papules Dif Yes SS b [11] SL W 14 2 Ch NS NS Erythematous, pruritic, maculopapular rash Dif Yes SS BB No [12] C3 W 16 SB 3 AA Crust NS Pruritic papulosquamous crusts Dif Yes c NS [13] SS W 17 4 My NS NS Papules, pustules, vesicles, and whitish crusts Dif Yes SS BB No [12] C5 W 5% 18 Inflammatory, pruritic scalp dermatosis with scale PC 5 To Scalp Yes Dif Yes SS No [14] C2 and erythema 1% W MS A Les HoF C R SSS Bur Secondary lesion morphology Dx Tx Recur Ref loc Inv G 20 Whitish, pruritic, keratotic crusts with almost total 6 In Crust NS Dif Yes SB NS d [12] C4 alopecia W

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Table 2 (continued). Characteristics of systemic lupus erythematosus patients who develop scabies infestationa. A R Les HoF C G SSS Bur Secondary lesion morphology loc Inv Dx Tx Recur Ref 10% 23 Piled-up, yellowish-white, scaly pruritic plaques SS S 7 Th Crust NS Dif Yes e [15] on a slightly erythematous base SB 3% W SA 30 Pruritic, crusting, erythematous, scaling plaques S& 25% 8 Th Crust NS Yes SB No [16] with diffuse scalp alopecia N BB W 34 Scaly, pruritic, erythematous, maculopapular rash 9 My Crust Yes Dif Yes SS f Yesg [12] C1 that progressed to thick, keratotic, crusts W 37 Sca 10 NS Crust NS Tender red plaque with adherent yellow scales Yes SB h No [17] lp W 37 Iver 11 Mi Scalp No Inflammatory dermatosis with scale and erythema Dif Yes SS No [14] C1 x 2 W 39 Nonpruritic erythematous, scaly papules and 1% 12 NS Incog No Dif Yes SB No [18] plaques LL W 40 13 Ch NS Yes Pruritic papular rash Dif NS SS BB No [12] C2 W 44 Iver 14 NS Crust Yes Confluent, scaling, crusted, pruritic plaques Dif Yes SS No [19] x 2 W 51 25% 15 NS Crust NS Pruritic, scaly, lichenified, crusted plaques Dif No SS BB No [20] W X 7d 11 Pruritic, maculopapular erythematous plaques 1% 16 Ma NS NS Dif Yes SS No [21] with lichenification LL M 46 SS 20% 17 NS Crust NS Pruritic, hyperkeratotic papules and plaques Dif Yes i [22] SB BB M aAbbreviations: A, age (years); AA, African-American; BB, benzyl benzoate; Bur, burrows; C, case; Ca, Caucasian; Ch, Chinese; Crust, crusted; d, days; Dif, diffuse; Dx, diagnosis of scabies infestation method; G, gender; HoF Inv, head or face involvement; Incog, incognito; Iver, ivermectin; Les loc, lesion location; LL, lindane lotion; Ma, Mexican-American; M, man; Mi, Maori; MS, malathion shampoo; My, Malay; NS, not stated; PC, permethrin cream; R, race; Recur, recurrence; Ref, reference; S, sulfur; S&N, scalp and neck; SA, salicylic acid; SB, skin biopsy; SL, sulfur lotion; SS, skin scrape; SSS, scabies surrepticius subtype; Th, Thai; To, Tongan; Tx, treatment; W, woman; x, times; &, and; %, percent. bDespite improvement in skin lesions, she died eight days later due to Pseudomonas aeruginosa septicemia and renal failure. cLesions were unresponsive to lindane and monosulphiran. Resolution required control of her systemic lupus erythematosus with extended treatment with corticosteroids. dThe skin biopsy wound became infected with Pseudomonas aeruginosa, leading to sepsis, and ultimately her death. ePatient died from a multifocal infection one week after starting treatment. fSupervised topical benzyl benzoate treatments for 48 hours on two occasions were unsuccessful. On the third trial, prolonged soaking in a bath, scrubbing off each individual crust, and 48 hours of benzyl benzoate provided resolution. gThere was recurrence of scabies three years after initial presentation. hPatient received ivermectin 0.2 mg/kg PO on days 1, 2, 8, 9, 15, 22, and 29. Permethrin cream was also applied nightly for seven days, then twice weekly for two weeks. She also was prescribed ciprofloxacin for Enterobacter superinfection. iPruritus resolved with treatment, but patient died several days later due to Staphylococcus aureus bacteremia.

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